[5] Most cases are identified in the first year of life with respiratory infections, hearing loss and intellectual disability.

Because of its rarity, and non-specific clinical findings, beta-mannosidosis can go undiagnosed until adulthood, where it can present with intellectual disability and behavioral problems, including aggression.

[7][2] Beta-mannosidase function is consistent with it being a lysosomal enzyme catalyzing and thus involved in degradation route for N-linked oligosaccharide moieties (glycoproteins).

[3] Diagnostic techniques for this condition can be done to offer a differential diagnosis, via lectin histochemistry, to distinguish between alpha-mannosidosis and beta-mannosidosis.

[9] There is currently no treatment available; individuals exhibiting muscle weakness or seizures are treated based on symptoms.